Baseline characteristics from the EXCITING-ILD registry

Background Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse.Methods The prospective Exploring Clinical and Epidemiological Characteristics of Interstitia...

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Main Authors: Buschulte, Katharina (Author) , Kabitz, Hans-Joachim (Author) , Hagmeyer, Lars (Author) , Hammerl, Peter (Author) , Esselmann, Albert (Author) , Wiederhold, Conrad (Author) , Skowasch, Dirk (Author) , Stolpe, Christoph (Author) , Joest, Marcus (Author) , Veitshans, Stefan (Author) , Höffgen, Marc (Author) , Maqhuzu, Phillen (Author) , Schwarzkopf, Larissa (Author) , Hellmann, Andreas (Author) , Pfeifer, Michael (Author) , Behr, Juergen (Author) , Karpavicius, Rainer (Author) , Guenther, Andreas (Author) , Polke, Markus (Author) , Höger, Philipp (Author) , Somogyi, Vivien (Author) , Lederer, Christoph (Author) , Markart, Philipp (Author) , Kreuter, Michael (Author)
Format: Article (Journal)
Language:English
Published: 25 December 2025
In: BMJ Open Respiratory Research
Year: 2025, Volume: 12, Issue: 1, Pages: 1-8
ISSN:2052-4439
DOI:10.1136/bmjresp-2024-002902
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1136/bmjresp-2024-002902
Verlag, kostenfrei, Volltext: https://bmjopenrespres.bmj.com/content/12/1/e002902
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Author Notes:Katharina Buschulte, Hans-Joachim Kabitz, Lars Hagmeyer, Peter Hammerl, Albert Esselmann, Conrad Wiederhold, Dirk Skowasch, Christoph Stolpe, Marcus Joest, Stefan Veitshans, Marc Höffgen, Phillen Maqhuzu, Larissa Schwarzkopf, Andreas Hellmann, Michael Pfeifer, Juergen Behr, Rainer Karpavicius, Andreas Guenther, Markus Polke, Philipp Höger, Vivien Somogyi, Christoph Lederer, Philipp Markart, Michael Kreuter
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Summary:Background Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse.Methods The prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases (EXCITING)-ILD registry included patients with all different ILD subtypes from different healthcare settings. Follow-up ranged from 36 months to 5 years. Data were analysed descriptively. Baseline characteristics, diagnostic and treatment information are presented as absolute numbers and percentages. The Wilcoxon signed-rank sum test was used to quantify differences between groups. Line plots and bar plots were used for graphical presentation.Results A total of 601 patients (60.7% men, mean age 64.3 years) from 32 centres were included in the EXCITING-ILD registry. The most common subtypes were sarcoidosis with 26.6% (n=160) and idiopathic pulmonary fibrosis (IPF) with 25.3% (n=152). Pulmonary hypertension was present in 8.7% of patients (n=52), with high incidences in connective tissue disease-associated ILD (16.3%) and pneumoconiosis (27.3%). The mean forced vital capacity was 76.4% predicted, and the mean DLCO-SB (diffusing capacity for carbon monoxide) was 54.1% predicted. The mean time to diagnosis was 38.8 months (SD 64.4) and was significantly shorter when the diagnosis was made after multidisciplinary discussion (31.6 vs 49.2 months, p<0.001). The frequency of surgical lung biopsies decreased over time in the registry, whereas the proportion of cryobiopsies showed a notable increase. In IPF, the number of patients treated with antifibrotics increased from 35.2% before 2015 to 48.4% in 2019.Conclusion The EXCITING-ILD registry describes the frequency of ILD subtypes, ILD-related impairments, selected comorbidities and diagnostic and treatment patterns in a representative German population.
Item Description:Gesehen am 31.03.2026
Physical Description:Online Resource
ISSN:2052-4439
DOI:10.1136/bmjresp-2024-002902